Cardiac Findings at Necropsy in Acute Type A Aortic Dissection.

Described herein are necropsy findings in 97 patients aged 22 to 82 years (mean 55), 37 women, 60 men, studied at necropsy with acute aortic dissection (AD) with the intimal-medial tear in the ascending aorta. The cases were studied from 1966 to 1989, a period when echocardiography and computed tomography were relatively infrequently available for diagnosis of AD. Arteriography was the method for diagnosis in most cases. Of the 97 cases, 30(31%) had operative intervention and 67 did not. Most appeared to have had systemic hypertension before the acute AD; only 4 had previous heart failure; only 8 had considerable atherosclerotic coronary disease; only 4 had a left ventricular (LV) scar and in each it was small; most (96%) had a normal-sized LV cavity (suggesting normal cardiac indices in them), and the other 4 had only a mildly dilated cavity; the heart weight in all 97 patients was increased; the quantity of subepicardial adipose tissue was increased in most patients, and the frequency of a congenitally malformed aortic valve was much higher than in the general population (6% - vs- 1%), but still uncommon. Thus, in > 90% of patients with acute Type A AD, coronary atherosclerosis was insignificant, myocardial fibrosis is absent, and the aortic valve has 3 cusps without stenosis.

Large patent ductus arteriosus in a 44-year-old woman leading to calcium deposition in the left atrium and mitral and aortic valves.

This report describes unusual autopsy findings in a 44-year-old woman who had a large, calcified patent ductus arteriosus that produced substantial left-to-right shunting. The patient died in 1962, 7 days after patch closure of the aortic orifice of the ductus. Numerous calcific deposits were present in the mural left atrial endocardium, the mitral valve leaflets and annulus, and the aortic valve cusps. The cause of the left-sided calcific deposits was perhaps related to the patient's several-decades-old giant aortopulmonary shunt, causing a major increase in the volume of blood passing through the left-sided cardiac chambers in comparison with the volume in the right side. To our knowledge, such findings in a patient with patent ductus arteriosus have not been reported previously.

Nontraumatic intracerebral hemorrhage unassociated with arterial aneurysmal rupture as a cause of sudden unexpected death.

Sudden death from intracerebral hemorrhage was observed in two patients admitted to Baylor University Medical Center at Dallas in a single month. Each had been drinking alcohol at the time of onset of first symptoms. Intracerebral hemorrhage was diagnosed in one patient by computed tomography, but not in the second patient who clinically was diagnosed as having acute coronary syndrome. Both died within 24 hours of onset of symptoms, and autopsy in both disclosed intracerebral hemorrhage, an infrequent cause of sudden death. This report calls attention to intracerebral hemorrhage as a cause of sudden death.

Clues to diagnosing carcinoid heart disease as the cause of isolated right-sided heart failure.

Described herein is a 67-year-old woman who underwent replacement of both tricuspid and pulmonic valves because of severe isolated right-sided systolic heart failure. The cause of the heart failure preoperatively was believed to be the result of left breast radiation a year earlier. At operation, however, the pulmonic valve was excised and a biopsy of the stiff-walled right atrium was performed, and histologic examination of each was classic of carcinoid heart disease. She never awoke postoperatively. Postoperatively, computed tomography disclosed numerous masses in the liver. Retrospectively, clues to the presence of carcinoid heart disease include thickening of both the tricuspid and pulmonic valve leaflets by echocardiogram, a pressure gradient, albeit small, across the pulmonic valve, the plastering of the septal tricuspid-valve leaflet to the ventricular septum, the total absence of left-sided heart disease, and the presence of extremely low 12-lead QRS electrocardiographic voltage.

Morphologic features of the recipient heart in patients having cardiac transplantation and analysis of the congruence or incongruence between the clinical and morphologic diagnoses.

Cardiac transplantation (CT) has been one of the great medical advances of the last nearly 50 years. We studied the explanted hearts of 314 patients having CT at Baylor University Medical Center Dallas from 1993 to 2012, and compared the morphologic diagnoses to the clinical diagnoses before CT. Among the 314 patients the morphologic and clinical diagnoses were congruent in 272 (87%) and incongruent in 42 (13%). Most of the incongruity occurred among the 166 patients with non-ischemic cardiomyopathy (non-IC) (36/166 [22%]), and of that group the major incongruity occurred among the patients with hypertrophic cardiomyopathy (7/17 [41%]), non-compaction left ventricular cardiomyopathy (NCLVC) (3/3 [100%]), mononuclear myocarditis (3/3 [100%]), arrhythmogenic right ventricular cardiomyopathy (ARVC) (4/4 [100%]), and cardiac sarcoidosis (8/8 [100%]). The phrase "non-IC" is a general term that includes several subsets of cardiac diseases and simply means "insignificant narrowing of 1 or more of the epicardial coronary arteries," but it does not specify the specific cause of the heart failure leading to CT. A number of cardiac illustrations are provided to demonstrate the morphologic variability occurring among the patients with IC and non-IC.

Dramatically different phenotypic expressions of hypertrophic cardiomyopathy in male cousins undergoing cardiac transplantation with identical disease-causing gene mutation.

Described herein are certain findings in 2 male cousins who underwent cardiac transplantation for severe heart failure (HF), one of the diastolic type (ejection fraction ≈65%), and one of the systolic type (ejection fraction ≈20%), both the consequence of hypertrophic cardiomyopathy (HC), and each had identical disease-causing gene mutations. The implanted heart in one had normal-sized ventricular cavities and no grossly visible ventricular wall lesions (except in one papillary muscle) and the other patient had severely dilated ventricular cavities and multiple extensive ventricular wall scars. The heart mass and the age of onset of symptoms was similar in each patient. A number of other family members had evidence of HC.

Effect of coronary bypass and valve structure on outcome in isolated valve replacement for aortic stenosis.

Reports differ regarding the effect of concomitant coronary artery bypass grafting (CABG) in patients who undergo aortic valve replacement (AVR) for aortic stenosis (AS), and no reports have described the effect of aortic valve structure in patients who undergo AVR for AS. A total of 871 patients aged 24 to 94 years (mean 70) whose AVR for AS was their first cardiac operation, with or without first concomitant CABG, were included. Patients who underwent mitral valve procedures were excluded. In comparison with the 443 patients (51%) who did not undergo CABG, the 428 (49%) who underwent concomitant CABG were significantly older, were more often male, had lower transvalvular peak systolic pressure gradients and larger valve areas, had lower frequencies of congenitally malformed aortic valves, had lighter valves by weight, had higher frequencies of systemic hypertension, and had longer stays in the hospital after AVR. Early and late (to 10 years) mortality were similar by propensity-adjusted analysis in patients who did and did not undergo concomitant CABG. Congenitally unicuspid or bicuspid valves occurred in approximately 90% of those aged 21 to 50, in nearly 70% in those aged 51 to 70 years, and in just over 30% in those aged 71 to 95 years. Unadjusted and adjusted survival was significantly higher in patients with unicuspid or bicuspid valves compared to those with tricuspid valves. In conclusion, although concomitant CABG had no effect on the adjusted probability of survival, the type of aortic valve (unicuspid or bicuspid vs tricuspid) significantly affected the unadjusted and adjusted probability of survival.

Cardiac transplantation in adults with aortic valve disease with focus on the bicuspid aortic valve.

The frequency of congenitally bicuspid aortic valves in patients having cardiac transplantation (CT) is unknown. We reviewed 243 explanted hearts in patients having CT at Baylor University Medical Center, Dallas from June 1997 through November 2011 to determine the frequency of a bicuspid aortic valve in this population. Of the 243 explanted hearts, 7 (2.9%) were found to have a congenitally bicuspid aortic valve: 3 had severe aortic valve stenosis and before CT had had the aortic valve replaced; the other 4 had normally functioning bicuspid valves and underwent CT for cardiomyopathy (ischemic in 1, idiopathic in 2, and hypertrophic in 1). Review of previously published reports of CT and aortic valve disease disclosed that 4 patients had had aortic valve replacement (AVR) from 2 to 8 years before CT, 3 had AVR or aortic valve repair of the donor heart at the time of CT, and 4 had AVR or transcatheter aortic valve implantation from 1 to 14 years after CT. Some of these aortic valve replacements, before, at the time of, or after CT were in patients with congenitally bicuspid aortic valves. In conclusion, congenitally bicuspid aortic valves were found in 7 of 243 explanted hearts in patients having CT at a single medical center in a 14-year period: 4 had functioned normally and 3 were severely stenotic. Previous reports of patients having AVR or repair before, during, and after CT were reviewed.

Effect of body mass index on survival in patients having aortic valve replacement for aortic stenosis with or without concomitant coronary artery bypass grafting.

The purpose of this report is to describe the effect of body mass index (BMI) on 30-day and late outcome in patients having aortic valve replacement (AVR) for aortic stenosis (AS) with or without concomitant coronary artery bypass grafting. From January 2002 through June 2010 (8.5 years), 1,040 operatively excised stenotic aortic valves were submitted to the cardiovascular laboratory at Baylor University Medical Center at Dallas. Of the 1,040 cases 175 were eliminated because they had a previous cardiac operation. The present study included 865 adults whose AVR for AS was their first cardiac operation. Propensity-adjusted analysis showed that 30-day and late mortality were strongly and significantly associated with BMI. Decreased risk of 30-day and long-term mortality was observed for patients with BMI in the low 30s compared to patients with BMI in the mid 20s or >40 kg/m(2). In conclusion, the findings in this study indicate a strong and significant adjusted association between BMI and 30-day and long-term mortality in patients having AVR for AS with or without concomitant coronary artery bypass grafting. Better survival was observed in patients with BMIs in the low 30s compared to patients with BMIs in the mid 20s and >40 kg/m(2).